Richard Bowman

Child eye health tertiary facilities in Africa

PURPOSEnThe World Health Organization and the International Agency for Prevention of Blindness recommends that there be 1 Child Eye Health Tertiary Facility (CEHTF) per 10 million people in developing countries. We sought to assess the current situation in Africa with regard to manpower, facilities, training, and output and to identify gaps in supportive services in CEHTFs.nnnMETHODSnA survey was conducted by sending comprehensive questionnaires to all CEHTFs across Africa. Of the 27 CEHTFs in Africa, 21 (77.8%) returned forms.nnnRESULTSnResults show significant variability of resources (manpower, training, infrastructure, equipment, and refractive services). The mean population catchment area of the CEHTFs was 9.42 million. In 2007 the mean number of surgeries for congenital and developmental cataract, strabismus, and other conditions was 72.0, 37.1, and 169, respectively, with wide variation between the centers. Overall there were 1.26 operated boys for every girl. The mean surgical fee charged was US

Global challenges in the management of congenital cataract: proceedings of the 4th International Congenital Cataract Symposium held on March 7, 2014, New York, New York.

117. The presence of a dedicated optometrist, childhood blindness coordinator, and a full-time anesthetist was associated with higher surgical output.nnnCONCLUSIONSnAlthough the 21 CEHTFs cover an estimated population of 197 million, the number of children receiving services was generally low. Findings suggest that an additional 20 sub-Saharan countries are not large enough to support a CEHTF, and some large countries require additional facilities. Strengthening existing CEHTFs would require investment in manpower (especially support by optometrists and childhood blindness coordinators), programs to identify and refer children needing services, and internally and externally supported mechanisms to support the relatively high cost of providing this service.

Cerebral visual dysfunction in prematurely born children attending mainstream school

Childhood cataracts have become a leading cause of preventable childhood blindness in many areas of the world. Here we summarize regional focus group discussions from the 4th Annual International Congenital Cataract Symposium on the current situation, challenges, and recommendations for the management of congenital cataracts in sub-Saharan Africa, the Middle East and North Africa, South Asia, Central America, South America, and developed nations. Strategies for managing congenital cataracts must be adapted and developed according to regional conditions. A basic framework for acceptable outcomes must focus on developing systems to address the critical components of education, access, quality care, and good follow-up.

Reliability of a question inventory for structured history taking in children with cerebral visual impairment

PurposeAlthough premature birth is recognised as a cause of cerebral visual impairment (CVI), which can include cerebral visual dysfunction (CVD), the incidence and nature of CVD in prematurely born children is not known.MethodsA prospective, controlled investigation was undertaken of forty-six, mainstream primary school children, prematurely born with gestations of 24.0–34.6xa0weeks, and of 130 control (term-born) children. Assessments were made of IQ, ophthalmic functions, visual perception and visual attention. Structured history-taking seeking evidence of behavioural features of CVI used a question inventory. Obstetric, neonatal and paediatric medical histories were documented from case records.ResultsFifteen out of forty-six (33xa0%) of the prematurely born children—“cluster A”—revealed behaviours corresponding with CVD on cluster analysis of the CVI inventory. The whole prematurely born group performed worse than controls on all visual perception tests and all four visual attention tests. Children in cluster A were responsible for this effect, performing worse than controls on all visual perception and visual attention tests except visual closure, while cluster B prematurely born children performed no differently to controls.ConclusionsThe prevalence of CVD in these prematurely born children is between 21–47xa0% (95xa0% CI), with a pattern similar to “dorsal stream dysfunction”. Currently available perceptual tests appear to be unable to identify the specific pattern of problems noted in this group. Many studies have provided evidence of cognitive and intellectual dysfunction in prematurely born children, and it is possible that CVD is a contributor. The CVI inventory is a potential means of identifying and characterising the condition, which can be ameliorated with simple strategies.

Childhood-onset Leber hereditary optic neuropathy

Thirty-six consecutive children presenting to our clinic over 12 months with problematic CVI (mean age 10.8, range 5–16.5 years, SD 3.1) and 156 children (mean age 8.24, range: 4.5–11.6 years, SD 1.92) from seven mainstream primary schools (excluding those with poor reading skills, dyspraxia, autism, and other developmental and behavioural disorders) comprised patients and controls.

First-year experience of chemotherapy for advanced retinoblastoma in Tanzania: disease profile, outcomes, and challenges in 2008.

Background The onset of Leber hereditary optic neuropathy (LHON) is relatively rare in childhood. This study describes the clinical and molecular genetic features observed in this specific LHON subgroup. Methods Our retrospective study consisted of a UK paediatric LHON cohort of 27 patients and 69 additional cases identified from a systematic review of the literature. Patients were included if visual loss occurred at the age of 12u2005years or younger with a confirmed pathogenic mitochondrial DNA mutation: m.3460G>A, m.11778G>A or m.14484T>C. Results In the UK paediatric LHON cohort, three patterns of visual loss and progression were observed: (1) classical acute (17/27, 63%); (2) slowly progressive (4/27, 15%); and (3) insidious or subclinical (6/27, 22%). Diagnostic delays of 3–15u2005years occurred in children with an insidious mode of onset. Spontaneous visual recovery was more common in patients carrying the m.3460G>A and m.14484T>C mutations compared with the m.11778G>A mutation. Based a meta-analysis of 67 patients with available visual acuity data, 26 (39%) patients achieved a final best-corrected visual acuity (BCVA) ≥0.5 Snellen decimal in at least one eye, whereas 13 (19%) patients had a final BCVA <0.05 in their better seeing eye. Conclusions Although childhood-onset LHON carries a relatively better visual prognosis, approximately 1 in 5 patients will remain within the visual acuity criteria for legal blindness in the UK. The clinical presentation can be insidious and LHON should be considered in the differential diagnosis when faced with a child with unexplained subnormal vision and optic disc pallor.

An ultrasound based classification of periocular haemangiomas

PURPOSEnTo examine the profile of retinoblastoma in a national tertiary referral center in Tanzania and to report first-year outcomes of its treatment using chemotherapy.nnnMETHODSnAll patients with retinoblastoma referred in 2008 were included. Disease was classified on clinical grounds as ocular, orbital, or metastatic. Those with ocular and orbital disease received chemotherapy. Remission was the main outcome measure and defined as absence of disease at the end of treatment.nnnRESULTSnIn 2008, 37 patients (20 males and 17 females) with retinoblastoma were referred to Ocean Road Cancer Institute. The mean delay from the first sign of disease to presentation at hospital was 10.4 ± 8.7 months. Disease was ocular in 32% (12 of 37), orbital in 57% (21 of 37), and metastatic in 11% (4 of 37). Of those with ocular disease, 67% (8 of 12) completed chemotherapy and all (8 of 8) achieved remission. In contrast, 48% (10 of 21) with orbital disease completed chemotherapy and only 50% (5 of 10) achieved remission. The difference in outcome between the groups was statistically significant (P = .001, Fisher exact test).nnnCONCLUSIONnThe profile of retinoblastoma in Tanzania is skewed toward severe invasive disease. Despite the introduction of chemotherapy, further improvements in mortality and morbidity can only be achieved through emphasis on early detection.

Off-Axis Digital Flash Photography: A Common Cause of Artefact Leukocoria in Children

Aims: To propose a classification system for periocular haemangiomas based on ultrasound evaluation. Methods: Retrospective review of ultrasound images from children seen in the authors’ unit with periocular haemangiomas. Static ultrasound images from 50 patients with periocular haemangiomas were reviewed as identified from a computerised database. Each haemangioma ultrasound image was classified into three categories: (1) preseptal only; (2) preseptal + extraconal; (3) preseptal + extraconal + intraconal. These were compared with the categories given to each patient at first presentation after dynamic scanning. Results: Classification was possible from the static images in 44 (88%) cases. Of those classified 20 (45%) were preseptal only; 17 (39%) were preseptal + extraconal, and seven (16%) had an additional intraconal component. The classification in all 44 cases was the same as that given at the time of presentation. In the small number of cases which went to surgery or had neuroimaging, the ultrasound classification was confirmed. Conclusions: Ultrasound classification was not difficult to perform and no child needed sedation or general anaesthesia for this exam. Ultrasound anatomical classification is an important first step in determining appropriate treatment of periocular haemangiomas. The authors present what they believe to be the first such classification.

Retinal nerve fibre layer thinning is associated with worse visual outcome after optic neuritis in children with a relapsing demyelinating syndrome

Leukocoria, a presenting sign in several significant pediatric ocular conditions, can be artefactually produced by off-axis flash photography in healthy eyes. The authors demonstrate the conditions needed to produce this phenomenon, which is more commonly seen in children due to their larger-sized pupils, photogenicity, and frequent off-axis shots.

Clinical utility gene card for: Wolfram syndrome

Optic neuritis may be monophasic or occur as part of a relapsing demyelinating syndrome (RDS), such as multiple sclerosis, aquaporin‐4 antibody (AQP4‐Ab) neuromyelitis optical spectrum disorder (NMOSD), or myelin oligodendrocyte glycoprotein antibody (MOG‐Ab)‐associated disease. The aims of this study were to test whether clinical, electrophysiological, and microstructural parameters differ in multiple‐sclerosis‐associated optic neuritis (MS‐ON) and antibody‐associated optic neuritis (Ab‐ON); to identify the clinical and paraclinical characteristics of children suffering worse long‐term visual outcome of RDS‐optic neuritis; and to explore the relationship between RNFL thickness and clinical parameters in RDS‐optic neuritis.